Objective: Papillary glioneuronal tumors (PGNT) is well-recognized in the literature, although reports usually have not attempted a critical analysis of their characteristics. We report two PGNT and perform a comprehensive review of the published cases, aiming to clarify their clinical, imaging and histopathological features.
Material and methods: We have reviewed all glioneuronal tumors diagnosed in our laboratory over the last 10 years and found 2 cases PGNTs along with their clinical, imaging and surgical data. We have processed material for light microscopy, and for immunohistochemistry study, we have used antisera against glial fibrillary acidic protein, Olig-2C, neurofilament protein, synaptophysin and Ki-67. We searched Medline (1966 through October 2007) for original articles or previous reviews.
Results: Case 1, a 19-year-old girl with a left, partially cystic, occipital tumor, totally removed, with no signs of recurrence 32 months after surgery, Case 2, a 9-year-old girl with a right, cystic with a solid nodule, temporal tumor, totally removed, with no signs of recurrence 19 months after surgery. Histopathology and immunohistochemistry studies favored a diagnosis of PGNT. A survey of 38 reported PGNT cases together with our two disclosed the following typical profile: young adulthood predominance, temporal lobe location, presence of cystic components: a close association with the lateral ventricles, a few anaplastic tumors, and gross total resections were usually possible with no recurrences the extent of surgical removal being the main prognostic factor.
Conclusions: Although histopathology is usually characteristic, imaging features may also be important in the presurgical evaluation of PGNTs. Gross total resections are usually possible and seem to govern prognosis. However, longer follow-up data are required.